POS1334 OCULAR CICATRICIAL PEMPHIGOID: IS THERE AN ASSOCIATION WITH AUTOIMMUNE DISEASES?
Authors: A. Ringer, A. M. Smichowski, R. Gomez, B. M. Virasoro, L. Martinez, E. Bertiller, C. Siegrist, B. Abdala, S. Chulibert, D. G. Grossi, E. Rubin, A. Kostianovsky, S. A. Munoz, I. Gandino
Journal: Annals of the Rheumatic Diseases (2022)

Background Ocular Cicatricial Pemphigoid (OCP) is an infrequent, systemic cicatricial immune-mediated disease, belonging to the group of membranous-mucosal pemphigoids (MMP). Due to the possibility of coexistence of multiple autoimmune diseases (ADs), OCP could be associated with other diseases. In the literature, association of OCP and ADs is reported in 9-35% of patients, but most reports correspond to MMP. Objectives To assess the prevalence of autoimmune diseases associated with OCP and to analyse clinical, laboratorial and treatment associations between these entities. Methods A multicentre cross-sectional study of patients with a diagnosis (clinical and/or by biopsy) of OCP derived from ophthalmology was performed. The population was divided into two groups according to their association or not with other ADs. Clinical, laboratorial and treatment variables were described and compared between both groups. In addition, a multivariate descriptive logistic regression analysis was performed to identify variables that could suggest the association between OCP and ADs. Results A total of 88 patients were recruited, 66 (75%) females, with a mean age at diagnosis of 64.3 years (SD 11.9). The median follow-up time was 1 year. The diagnosis was done by biopsy in 86,8%. Ocular bilateral disease was present in 95,3% of patients. There was a median delay from symptoms onset to diagnosis of 2 years. A history of malignancy was reported in 13,6%. Extraocular involvement was evidenced in 11,5% (4% compromised skin and 9,1% other mucous membranes). Regarding the previous clinical findings, no statistically significance was found between the groups with and without ADs. Systemic treatment was depicted as follows: oral corticosteroids (60,2%) ( p -value &lt; 0.001), corticosteroids pulses (5,7%), dapsone (3,4%), methotrexate (79,5%), mycophenolate (15,9%), azathioprine (23,9%), rituximab (5,7%), immunoglobulin (1,1%); topical corticosteroids (96,6%) and ocular infiltration (2,3%). The group associated ADs included 24 patients (27.3%). Within them, the most prevalent diagnosis was Sjogren’s syndrome (13.6%), followed by Hashimoto´s thyroiditis (9,1%) and rheumatoid arthritis (3,4%). Most of the patients presented Foster stages 1 (45.3%) and 2 (29.3%) at diagnosis. In the ADs group, statistically significant associations were observed with ANA, SS-A and SS-B antibodies, rheumatoid factor, and hypergammaglobulinemia. In the descriptive multivariate logistic regression model, it was detected that hypergammaglobulinemia was associated with ADs and OCP, adjusted for age, sex, smoking, skin and mucosal involvement, and erythrocyte sedimentation rate (OR 8.7; 95% CI 1.6 to 46.8; p = 0.012), Table 1. Table 1. Multiple logistic regression analysis with OCP associated with ADs as dependent variable. OR CI95 P value Gender 0.2 0.04 - 1.1 0.07 Age at diagnosis 1.02 0.9 - 1.1 0.25 Smoking 0.5 0.1 - 1.7 0.26 Skin and mucosa compromise 1.2 0.2 - 8.2 0.83 ESR 0.98 0.95 - 1.02 0.58 Hypergammaglobulinemia 8.7 1.6 - 46.8 0.012 ESR: Erythrocyte Sedimentation Rate. OR: Odd Ratio. CI95: confidence interval Conclusion Due to the autoimmune nature of OCP, it could coexist with other ADs. In this research, it was observed that more than a quarter of the population presented with such association and hypergammaglobulinemia could suggest it. A systematic search for this coexistence should be carried out to avoid sequelae or incomplete treatment in pathologies that are currently potentially treatable. References [1]Lamberts A et al. European guidelines on diagnosis and management of mucous membrane pemphigoid, initiated by the European Academy of Dermatology and Venereology – Part I. 2021;1750–64. [2]Schmidt E et al. European Guidelines on diagnosis and management of mucous membrane pemphigoid, initiated by the European Academy of Dermatology and Venereology – Part II. 2021;1926–48. [3]Narla S, Silverberg JI. Associations of pemphigus or pemphigoid with autoimmune disorders in US adult inpatients. J Am Acad Dermato. 2019;15-25. Acknowledgements On behalf of the Study Working Group of Rheumatological Ocular Diseases, Argentinian Society of Rheumatology. Disclosure of Interests None declared